Takayasu's arteritis (TA) is a chronic large-vessel arteritis in which the aorta, its major branches, and the pulmonary arteries are affected. The clinical signs of TA are insidiously varied, depending on the arterial sites involved. In this paper, we report an unusual presentation of TA with signs and symptoms of heart failure and the involvement of the renal artery in a 34-year-old male who was managed with medical treatments and an interventional procedure. Given the unusual presentation of TA, we recommend a high clinical index of suspicion of renal artery involvement and sufficient heed to its significance especially in view of the fact that its early diagnosis and timely appropriate treatment can confer a much better prognosis.

Takayasu's arteritis (TA) is a chronic large-vessel arteritis which affects the aorta, its major branches, and the pulmonary arteries. The course of the disease may be in tandem with segmental stenosis, occlusion, dilatation, or aneurysm.[1],[2] TA could involve all large arteries; however, the ones most affected are the ascending and descending aortas, subclavian arteries, and extracranial arteries including the carotids (60%–90%).

Case Report

A 34-year-old male, a known case of TA, was admitted in our hospital with flash pulmonary edema, pleural effusion, anuria, and an elevated creatinine level. The patient's workup showed a pulse rate of 104 beats/min, cardiomegaly and signs of cephalization on chest X-ray [Figure 1], and sinus tachycardia on electrocardiography. He underwent dialysis because of a progressive rise in the creatinine level and the deterioration of the symptoms.

The patient's history showed a previous hospitalization due to dyspnea, during which he suffered the onset of heart failure symptoms. Standard heart failure therapy was prescribed to lower his severe left ventricular dysfunction as well as elevated levels of erythrocyte sedimentation rate, C-reactive protein, and pro-brain natriuretic peptide. He underwent cardiovascular magnetic resonance imaging, which revealed that he had all of the three tissue characterization criteria for myocarditis as follows: edema, hyperemia, and scar [Figure 2]. Specific laboratory tests and the collected data showed that his cardiac dysfunction was related to TA. Although he received steroid pulse therapy as part of his medical heart failure treatment, an echocardiographic examination demonstrated marked dilatation and generalized hypokinesia in the left ventricle (ejection fraction = 25%). No significant valvular lesions were detected.

In the recent hospitalization, an evaluation of the patient's kidneys with transabdominal sonography showed a normal size and no significant stenosis at the visible part. In spite of the normal sonography of the renal arteries, renal angiography was scheduled for better evaluation of renal artery. The procedure illustrated a stenosis at the ostium of the left renal artery that was successfully stented subsequently [Figure 3]. Stenting was done through the right femoral access after the wiring of the left renal artery with a balance middleweight 0.014 wire and the predilation of the significant ostial stenosis with a 3.25 mm × 15 mm Hiryu. Postdilation was performed with a 6.0 × 18 RX Herculink Elite stent and a 5.5 mm × 20 mm LitePAC balloon [Figure 4].

At 1-month follow-up, the patient underwent angiography for an examination of the right renal artery, which showed that the artery was completely occluded from ostium.

Patient consented to publish his case anonymously by filling in the consent form.

Discussion

Currently, there is not enough data on TA in the existing literature probably due to the rarity of the disease. These limited data, however, indicate that despite the worldwide distribution of TA, it appears to be much more common among the Asian population inasmuch as most of the relevant evidence comes from this continent. Two epidemiologic studies, one from East Asia and the other from West Asia, estimated the incidence of TA at 1–2 per million in Japan and 2.2 per million in Kuwait.[3] Chiming in with those investigations, previous research shows that the incidence rate of TA in Iran is not very low.[4]

The symptoms and the systemic signs of TA are usually nonspecific because at the early clinical stages of the disease not only are the clinical signs insidiously diverse but also they are subject to the arterial sites involved. The left subclavian artery is the most frequently affected site in TA, with the aorta, the common carotid artery, the renal artery, and the vertebral artery constituting the other sites that are more likely to be affected. TA is associated with the renal artery injury in between 30% and 35% of the cases. The majority of the cases of renal artery stenosis are induced by atherosclerosis or fibromuscular dysplasia,[5] and hypertension resulting from renal artery stenosis is usually observed in children under 18, among whom the stenosis of the renal artery accounts for 10% of all the etiologic causes of hypertension. The vast majority of TA studies have concentrated on brachiocephalic involvement, with no reports on the real impact of renal stenosis. Nevertheless, it is well established that TA can give rise to hypertension and fatal cardiovascular problems. Stenosis is present in between 23% and 31% of the involved renal arteries, while other lesions such as occlusion, dilation, and aneurysm, are often more common. The stenosis of the renal artery induced by TA might lead to malignant hypertension, severe renal damage, cardiac decompensation, and even early death.[5] Arterial hypertension is seen in 33%–83% of patients suffering from TA, with renal artery stenosis contributing to this factor in 20%–38% of the cases. In addition to considerable morbidity, a 5-year TA-related mortality rate of 35% has been reported previously. This arterial disease is still a clinical challenge at all stages, and its treatment decisions are hindered by the lack of sufficient evidence for and against the designated therapies. Steroid treatment alone is not enough to avoid vasculitis progression in about 50% of the cases, necessitating the addition of immunosuppressive therapy. The difficulty in treating patients with TA is a reflection of the nature of the disease and its rarity, which limits the possibility of upcoming clinical trials.[5] Saddekni et al.[6] performed percutaneous transluminal angioplasty on patients suffering from TA with renal involvement for the first time in 1980. Since then, this procedure has been performed successfully several times and become the alternative treatment to the surgical approach with better results in pediatric patients.[7]

To sum up, TA with renal artery involvement must be regarded as an etiologic factor of secondary hypertension even when no blood pressure difference between the extremities is present. According to previous studies, the first presentation of TA could appear in the form of heart failure symptoms. Aortic valvular disease, systemic hypertension, and hemodynamic impacts of the coronary artery involvement have usually been associated with congestive heart failure.

In light of the above-mentioned evidence, we suggest that more attention be paid to the importance of renal artery involvement in patients with TA because an early diagnosis and a proper timely treatment can confer a significantly better prognosis. Nonetheless, left ventricular dysfunction resulting in congestive heart failure has been witnessed in patients with TA lacking these hemodynamic elements.[8] Given that TA could be a fatal disease, all cases with suspected TA or already diagnosed with TA should be examined for the involvement of all the organs – particularly the heart and the kidney.